You need to get calories from somewhere, should it be from carbohydrate or fat?

Saturday, January 17, 2015

GSD IIIa, ketones, MAD and Veech again

I mentioned the high protein/exogenous ketone approach to Glycogen Storage Disease IIIa in a recent post. It's very nice that an effective treatment can actually be achieved through a Modified Atkins Diet (MAD at <10g of carbs per day) involving food, w/o faking ketosis though those exogenous synthetic ketones.

Robert gave me the heads up on the latest paper on GSD IIIa using MAD, available as a free text through Pubmed. It's rather good as, again, it shows that there are medics out there who think matters through and occasionally come to correct conclusions. I love the clinical details of compliance/non compliance too. And that the early hypoglycaemia, treated with corn starch (bleugh), was asymptomatic under ketosis.

which is a fascinating personal insight in to what it was like to work in Hans Krebs' lab, combined with the sort of hard core math which implies rather more understanding of biochemistry than simply adding MitoSOX red to some cells and looking for colour changes to show oxidative stress. That's some complex reading to work through when clinical and home life combine to give me a chance!

20 comments:

Anecdote re: hypoglycemia in ketosis, a few days ago I experimented with lowering protein intake, with expected result of higher ketones from less glucose. My sugar often runs low I'm keto without symptoms,b but two days ago I noted particularly purple pee and was curious what my sugar was, and discovered it was 48. I checked my blood ketones at that time and they were only 1.3.

In spite of blood sugar of 48 and ketones only of 1.3, I had no symptoms at all of neuroglycopenia.

I consider this evidence of following1.protein is an important source of blood sugar2.keto adaption involves significant direct use of ffa for energy both body and brain, as low blood sugar and low ketones peripheral dis not produce symptomatic hypoglycemia. My brain ketones were likely higher from ffa transport being increased, whereas my body was oxidizing ffa directly for energy...thus, no sx in spite very low blood level of nutrients.

Hi Wooo, that's cool. I spent so long on the hyperglycaemia paper trying to see the logic to down regulating pancreatic glucokinase in fasting i.e. metabolism is quite happy for a day or two of hypoinsulinaemic hyperglycaemia when mixed food becomes available at the end of a period of extended fasting. But hyperglycaemia combined with hyperinsulinaemia appears verboten physiologically and bad in the paper. But the paper never quite lets you see in to this clearly.......... BTW I'm not very good at commenting on the net but Scribble Pad is compulsory reading

Peter the redox potential is going to be the next key metric in medicine. University of Virginia heart surgeons are now using it as the main indicators of for and against surgery and post operative therapies. The issue is how best to measure it clinically. Right now we have non specific ways to do it clinically and this is how I use labs to draw inferences to the intracellular state. It determine how biochemistry unfolds in us.

I discovered recently an entry in one of my microprocessor blogs about cell biology. It was quite surprizing, because the blogger has nothing to do with metabolism and health stuff, he's a real hardware engineer of exceptional knowledge.He doesn't pretend to know anything about cells, but he was fascinated by his misconceptions in face of articles he read.He presents several animations and factoids who are really surprizing when viewed from an engineers point of view (the proton pump being in fact an electric motor with a speed of 40000 rpm and such).Interesting read.

I get good results with one of the simple 1 litre $30 makers you can find on ebay. I take 800ml whole milk (Paris Creek unhomogenised, at a shop near you in SA) and add 200 ml of pure cream and brew it overnight.

btw I was amused by your experiences with bamboo, I have a similar photo of a trailer full of arundo donax roots.

Peter, Dr. Bernstein states in his internet talk, from December 14, 2014, here is the link,

http://attendthisevent.com/?eventid=63214041

in answer to a question from a Type 1 diabetic: "Could eating butter raise blood sugar, due to fatty acids in the blood causing the liver to convert protein to sugar? (at 29:55 on the MP3 audio recording),

Dr. Bernstein states that when there are high levels of FFAs in the blood, that the liver sees, that eating butter might cause the blood sugar to increase, the liver gets permission to convert amino acids to glucose, and, also the blood sugar could rise due to the incretin effect ("the Chinese Restaurant Effect"), when GLP1 (Glucagon-like-Peptide) tells the pancreas to make insulin and glucagon. If the diabetic cannot make insulin, only glucagon will be produced.

Dr. Bernstein said on the recording, that this question was the most intelligent question he'd seen in a long time.

George, I started taking all my cream and butter in cultured/fermented form, avoiding sweet cream and butter, and hard cheeses. As a result of this, many of my food allergy/intolerance symptoms disappeared.

I use Homestead Creamery half-'n'-half and their heavy whipping cream, which are pasteurized, not ultrapast., and not homogenized. That's the closest to raw cream I can get where I live.

Peter posted some years ago about several health troubles clearing up, when taking all his cream in fermented/cultured form. That inspired me. :)

Took me a while to give up the sweet cream and butter, etc., but it was worth it.

I made fermented cream in the Easy Yo for about four years and in the end time pressure, mostly, retired it. I liked the ability to adjust the acidity obtained (mostly the more the better). Down side was the occasional contaminant culture. But with the hours I work nowadays just keeping the chickens looked after doesn’t leave much to spare!

My children still drink fresh milk and the bugs in non pasteurised milk from grass fed Jersey cows seems to suit them quite well. I drive past this place every day, except when I stop to buy milk of course:

http://www.the-calf-at-foot-dairy.co.uk

I've left a litre at room temp to ferment in its own bugs in the past and it makes a nice buttermilk type drink, if a little high in protein for my preferences, so not often.

It seems you are lucky to have access to such a well-run dairy. Our laws in the penal colony are heading further in the opposite direction.

http://tinyurl.com/q9pa9gn

A toddler died late last year from haemolytic uraemic syndrome, apparently. The coronial inquest hasn't been finalised, but the pollies are not known for waiting until all facts are known. That would be too logical.

I had a neighbour who supplied fresh goat milk to most of the Adelaide metro area. The powers-that-be made him print "Warning Contains Raw Unpasteurised Goat Milk" on the containers in letters so large that you couldn't read the rest of the label. Obviously it is much more dangerous than smoking.

About Me

I am Petro Dobromylskyj, always known as Peter. I'm a vet, trained at the RVC, London University. I was fortunate enough to intercalate a BSc degree in physiology in to my veterinary degree. I was even more fortunate to study under Patrick Wall at UCH, who set me on course to become a veterinary anaesthetist, mostly working on acute pain control. That led to the Certificate then Diploma in Veterinary Anaesthesia and enough publications to allow me to enter the European College of Veterinary Anaesthesia and Analgesia as a de facto founding member. Anaesthesia teaches you a lot. Basic science is combined with the occasional need to act rapidly. Wrong decisions can reward you with catastrophe in seconds. Thinking is mandatory.
I stumbled on to nutrition completely by accident. Once you have been taught to think, it's hard to stop. I think about lots of things. These are some of them.

Organisation (or lack of it)!

The "labels" function on this blog has been used to function as an index and I've tended to group similar subjects together by using labels starting with identical text. If they're numbered within a similar label, start with (1). The archive is predominantly to show the posts I've put up in the last month, if people want to keep track of recent goings on. I might change it to the previous week if I ever get to time to put up enough posts in a week to justify it. That seems to be the best I can do within the limits of this blogging software!